Case Presentation: A 54-year-old man with a past medical history of intravenous drug use and morbid obesity presented with worsening bilateral lower leg pain and swelling. The patient reported chronic bilateral leg edema after a car accident a year before and started noticing skin changes with multiple bumps on his legs for several months. He reported multiple diagnoses of cellulitis, which were treated with antibiotics. The patient denied any recent traveling or animal bites. Review of systems was negative for orthopnea or shortness of breath. He did not seek care for the leg edema or skin changes since the COVID-19 pandemic started. He mentioned that he lost insurance coverage during this period, which also prevented him from seeking medical care. Physical examination revealed diffused verrucous plaques to bilateral lower legs with chronic edematous changes, dorsal feet with yellow serum discoloration, and white malodorous maceration between the toes. Laboratory studies showed WBC of 5,000 cells/mL, albumin 1.7 g/dL, CRP 291, and ESR 100. Echocardiogram was normal. CT of bilateral legs showed no signs of abscess or focal fluid collection. The ankle-brachial index was unremarkable. Dermatology was consulted. These findings were consistent with Elephantiasis Nostras Verrucosa (ENV) a complication from secondary lymphedema and superimposed soft tissue infection. The patient was started on ceftriaxone and vancomycin, topical econazole, gentamicin cream, and acetic acid soak. Additionally, a pneumatic lymphedema device was provided before discharge, along with wound care and dermatology follow-up.
Discussion: Lymphedema affects about 250 million patients worldwide. In developed countries, the majority of cases of lymphedema are secondary to cancer or its treatment.1 The primary complications of lymphedema include poor wound healing, increased risk of infection, pain, limited movement, and in rare cases, the development of cancers. We presented a case of Elephantiasis Nostras Verrucosa, a rare complication of chronic non-filarial lymphedema that causes progressive cutaneous hypertrophy. ENV is characterized by nonpitting edema and superimposed hyperkeratotic papulonodules with a verrucose or cobblestone-like appearance. 2 The pathogenesis of ENV is still unclear. The diagnosis is mainly based on history, physical exam, and typical cutaneous lesions similar to our patient. It is crucial to identify the cause of ENV, which could be from morbid obesity, prior surgery or trauma, congestive heart failure, deep vein thrombosis, or malignancies such as lymphoma.2 Our patient’s workup for the etiologies is negative, but he has multiple risk factors, including previous history of trauma, morbid obesity (BMI 40.1), and hypoalbuminemia. Initiating treatment during the early stage will affect survival outcome. In this case, a lack of early access to healthcare services affected our patient’s condition, leading to ENV with poor wound healing.
Conclusions: This case illustrates a rare complication of chronic lymphedema, which can be managed but is generally not cured. This condition is often challenging to treat, particularly if it is the later stage. Social determinants of health played a huge role in the outcomes of our patient. These include funding status, inability to access care during an ongoing pandemic, and poor health literacy. This cautionary case emphasizes the importance of early diagnosis and intervention, which play a significant role in developing chronic morbidity and impacting quality of life.
